AB1269 SHULMAN’S DISEASE OR EOSINOPHILIC FASCIITIS, A RARE FIBROSING DISORDER: A CASE REPORT TREATED WITH TOCILIZUMAB
Authors: A. Laria, A. M. Lurati, M. G. Marrazza, K. Re, D. Mazzocchi, P. Faggioli, A. Mazzone
Journal: Annals of the Rheumatic Diseases (2022)

Background Schulman eosinophilic fasciitis (EF) is a rare fibrosing disorder, typified by erythema, edema, and symmetric induration of the distal bilateral extremities (only proximal to the wrists and ankles) or/and trunk (1). The “groove sign” and/or a pseudocellulite appearance of the involved skin may be present (1), followed by joint contractures (1). Fascial biopsy is the gold standard for making a diagnosis.Systemic corticosteroids are first-line treatment (2). The use of c/bDMARDs is described in literature with clinical improvements (2). Objectives to evaluate efficacy of Tocilizumab in a patient affected by Shulman’s disease Methods A 54-year-old woman went to our observation for appearance of abrupt-onset simmetric edema followed by induration of the bilateral extremities and trunk. She has been feverish for about 2 weeks associated with diffuse arthromyalgia. On rheumatological examination: non-liftable skin in folds on the bilateral arms and forearms and lower limbs; absence of frank synovitis or lymphoglandular swellings. The blood tests performed showed an increase in acute phase reactants. The ferritin and electrophoretic picture, complement and immunoglobulin dosage were normal. The blood count showed a marked absolute eosinophilia accompanied by modest monocytosis and modest platelet disease. There was a slight increase in LDH and in serum beta2microglobulin. Serology for viral infections and autoimmunity were negative. Screening for celiac disease was negative. Coprocultures, parasitology and the search for the fecal H. pylori antigen are negative. Excluded lymphadenopathy or hepatosplenomegaly to the total body computed tomography. Modest non-buffering pericardial effusion was reported in CT scan. Nailfold capillary changes were absent. Chronic inflammatory bowel diseases were excluded. A skin biopsy of the left forearm confirmed the clinical diagnosis of EF. In particular, it documented thickening of the fascia, site of an inflammatory process that included small lymphocytes, plasma cells, histiocytes and some eosinophils, distributed both diffusely and around blood vessels.The underlying muscle tissue and the supra-fascial skin were free from alterations. Secondary fasciitis was excluded by 18 fluorodeoxyglucose (FDG)-positron emission tomography; this showed a diffusely accumulation of the radiopharmaceutical in the muscles of the forearms and of both lower limbs, compatible with eosinophilic fasciitis and confirmed the pericardial effusion. Immunophenotypic analysis on peripheral blood excluded the presence of a monoclonal cell population. Diagnosis of primary eosinophilic fasciitis was made and therapy was started with methylprednisolone 1 g intravenously for 3 days followed by another 3 boluses of methylprednisolon of 500 mg iv. Given the poor response, it was decided to set up therapy with Methotrexate 15 mg up to a dose of 20 mg intramuscularly per week associated with prednisone 25 mg/day. The rheumatological re-evaluation at 3 months showed a worsening of the fibrosis and diffuse skin edema with an obligatory kyphosis of the spine and limitation in the patient’s movements. Therefore it was decided to start off label infusion therapy with Tocilizumab 8 mg/kg/month. Results A complete resolution of the skin picture with disappearance of the pericardial effusion was documented 12 months after the start of the biological drug Conclusion There are currently no guidelines for the treatment of eosinophilic fasciitis. Tocilizumab has been shown to be effective when introduced in the early inflammatory stages of the disease, as in the case described. References [1]Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. 1988;17(4):221–31. [2]Daniel R. Mazori &amp; Alisa N. Femia &amp; Ruth Ann Vleugels. Eosinophilic Fasciitis: an Updated Review on Diagnosis and Treatment Curr Rheumatol Rep (2017) 19: 74. Disclosure of Interests None declared